The present invention relates to a pharmaceutical formulation suitable for parenteral administration containing carglumic acid and a buffering agent having a pK a from 5.5 to 9.0 at 25°C; according to an embodiment, the buffering agent may have a pK a from 7.5 to 8.5, preferably a pK a of about 8.07, such as trometamol. The formulation may also contain at least one bulking agent, such as mannitol. The invention also includes a method for manufacturing a lyophilised sterile formulation by freeze-drying a water solution containing carglumic acid, a buffering agent having a pK a from 5.5 to 9.0 at 25°C, preferably from 7.5 to 8.5, and optionally a bulking agent to obtain a freeze-dried powder.